Paraneoplastic autoantibodies (PNAs), relevant autoantigens, characteristic staining patterns, and associated paraneoplastic neuronal disorders (PNDs) and tumours
| PNA | Neuronal auto antigen(s) | Immunocytochemical staining pattern | PND | Tumour |
|---|---|---|---|---|
| ACh, acetylcholine; BE, brainstem encephalitis; CNS, central nervous system; LE, limbic encephalitis; LEMS, Lambert-Eaton myasthenic syndrome; MG, myasthenia gravis; PCD, paraneoplastic cerebellar degeneration; PEM, paraneoplastic encephalomyelitis; PNA, paraneoplastic neuronal autoantibody; PND, paraneoplastic neurological disorder; POM, paraneoplastic opsoclonus–myoclonus; PSN, paraneoplastic sensory neuronopathy; SCLC, small cell lung carcinoma; SPS, stiff person syndrome. | ||||
| Anti-Hu (ANNA-1) | 35–40 kDa neuron specific RNA binding proteins | All neuronal nuclei including myenteric plexus, weaker cytoplasmic staining | PEM, PCD, PSN | SCLC, other carcinomas |
| Anti-Ri (ANNA-2) | 55 and 80 kDa RNA neuronal binding protein | All CNS neuronal nuclei with nucleolar sparing, not DRG neurones (in contrast to Hu) | POM, PCD, BE, | Breast, SCLC, gynaecological |
| ANNA-3 | 170 kDa | Purkinje-cell cytoplasm and nucleus; renal glomerular podocytes | LE, PCD, PSN | SCLC, oesophageal |
| Anti-Yo (PCA-1) | 34 and 62 kDa DNA binding, gene transcription regulators | Purkinje cell cytoplasm and axons | PCD, peripheral neuropathy (rare) | Breast and gynaecological, oesophageal |
| Anti-PCA 2 | 280 kDa | Purkinje-cell cytoplasm and other neurones | PEM, PCD, LEMS | SCLC |
| Anti-Ma 1 | 40 kDa | Nucleus | BE, PCD | Lung cancer |
| Anti-Ma 2 (Ta) | 41.5 kDa | Nucleus, perikaryon | LE, BE | Testicular cancer |
| Anti-Tr | Unknown antigen | Purkinje cell cytoplasm, fine granular staining in molecular layer; all nuclei of CNS | PCD | Hodgkin’s disease |
| Anti-mGluR1 | Metabotropic glutamate receptor | Purkinje cell cytoplasm, climbing fibres | PCD | Hodgkin’s disease |
| Anti-amphiphysin | 128 kDa synaptic vesicle protein | Presynaptic nerve terminals of CNS | SPS, PEM | Breast, SCLC |
| Anti-VGKC | Voltage gated potassium channel | Peripheral nerve | Neuromyotonia | Thymoma, SCLC |
| Anti-VGCC | 64 kDa P/Q voltage gated calcium channel | Pre-synaptic neuromuscular junction | LEMS, PCD | SCLC |
| Anti-ACh R | ACh receptor | Post-synaptic neuromuscular junction | MG | Thymoma |